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[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

Olfactory neuroblastoma is an unusual neuroectodermal malignancy, which is thought to arise at the olfactory membrane of the sinonasal tract.

The aim of the current study is to identify specific DNA copy number changes in olfactory neuroblastoma.

Our results show that gene copy number profiles of olfactory neuroblastoma samples are complex.

Gains were more frequent than losses, and high-stage tumors showed more alterations than low-stage olfactory neuroblastoma.

In addition, our results indicate that gains in 20q and 13q may be important in the progression of this cancer, and that these regions possibly harbor genes with functional relevance in olfactory neuroblastoma.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

Three cases of olfactory neuroepithelioma are presented in this report.

Ultrastructurally, the tumor cells had the differentiation features of olfactory epithelium.

Olfactory neuroepithelioma is a rare occurrence and it can be very difficult to distinguish olfactory neuroepithelioma from small cell carcinoma, neuroendocrine carcinoma and so-called "olfactory neuroblastoma" on the basis of hematoxylin and eosin stained sections alone.

In controversial cases, a diagnosis of olfactory neuroepithelioma must be substantiated by ultrastructural and immunohistochemical findings, particularly regarding the detection of Ber-EP4 and LH-RH immunoreactivity.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] [Magnetic resonance imaging of olfactory neuroblastoma].

Despite the development of electron microscopy and immunohistochemical testing, the pathological diagnosis of this tumor is still difficult because of the wide range of histological features.

The purpose of this report was to analyze the MR characteristics of olfactory neuroblastomas.

Seventeen patients with olfactory neuroblastoma were treated at Hokkaido University Hospital and a related hospital during the past 25 years.

MR images taken in 12 patients and CT images taken in 9 patients with histologically confirmed olfactory neuroblastoma were retrospectively reviewed.

In most cases, olfactory neuroblastomas are hypointense on T1-weighted images, hyperintense on T2-weighted images, and show marked homogeneous enhancement with well-demarcated regular margins upon gadolinium enhancement.

Although the definite diagnosis is based on histopathology findings and MR features are nonspecific, they may suggest an imaging diagnosis of olfactory neuroblastoma when seen in the superior nasal cavity.

MR is superior to CT both in delineating the extent of the tumor and in making an imaging diagnosis.

Biopsies obtained via rhinoscopy led to a diagnosis of olfactory neuroblastoma.

Clinical signs are variable, and diagnosis may prove challenging as this tumor, which is a differential for spontaneous intranasal tumors in cats, may be mistaken for poorly differentiated carcinomas or round cell tumors.

Given its rarity, treatment options for this neoplasm have not been thoroughly evaluated and there is no standard treatment protocol.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

BACKGROUND: Cranionasal resection was first described in 1997 for the surgical resection of olfactory neuroblastoma.

The authors have routinely practiced cranionasal resection since 1996 for resection of all anterior skull base tumors in which the resultant skull base bony defect is limited to the nasal and sinus roof.

The aim of this study was to review the results of cranionasal resection for olfactory neuroblastoma.

METHODS: The results of cranionasal resection for olfactory neuroblastoma in six patients from 1996 to 2003 were reviewed.

Postoperative radiotherapy was given only to the patient with Kadesh stage C disease.

Postoperative radiotherapy is not necessary after clear resection of Kadesh A and B tumors.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Proton-beam therapy for olfactory neuroblastoma.

PURPOSE: To analyze the feasibility and efficacy of proton-beam therapy (PBT) for olfactory neuroblastoma (ONB) as a definitive treatment, by reviewing our preliminary experience.

Olfactory neuroblastoma is a rare disease, and a standard treatment strategy has not been established.

Radiation therapy for ONB is challenging because of the proximity of ONBs to critical organs.

METHODS AND MATERIALS: A retrospective review was performed on 14 patients who underwent PBT for ONB as definitive treatment at the National Cancer Center Hospital East (Kashiwa, Chiba, Japan) from November 1999 to February 2005.

One patient died from disseminated disease.

Liquorrhea was observed in one patient with Kadish's stage C disease (widely destroying the skull base).

CONCLUSIONS: Our preliminary results of PBT for ONB achieved excellent local control and survival outcomes without serious adverse effects.

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(PMID = 16802140.001).

[ISSN] 0937-4477

[Journal-full-title] European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] [Clinicopathologic study of sinonasal teratocarcinosarcoma and its contrast with olfactory neuroblastoma].

OBJECTIVE: To study the clinicopathologic features, diagnosis and differential diagnosis of sinonasal teratocarcinosarcoma (SNTCS) and olfactory neuroblastoma (ONB), and to discuss the histogenesis and possible relationship between SNTCS and ONB.

METHODS: Seven cases of SNTCS and 34 cases of ONB were retrieved from the pathological archives together with one case each of malignant teratoma and immature embryonic tissue at 8 weeks were collected from Beijing Tongren Hospital.

In addition, carcinoid, and primitive mesenchymal tissue and the ONB component were also seen.

METHODS: Totally 40 cases of ONB were studied using histology and immunohistochemistry techniques.

All the cases of ONB were graded according to Hyams Grading system.

In addition, 3 cases showed an in-situ form with invasion of tumor into olfactory epithelium, and there was exogenous papillary proliferation seen in 2 cases.

CONCLUSIONS: Unusual pathological structures including epithelial structures, in-situ invasion of tumor tissue into the involving olfactory epithelium and exogenous papillary proliferation can be found in ONB, suggesting that ONB may originate from the undifferentiated basal cells of olfactory epithelium, through bipotential differentiation.

The presence of tumor necrosis in ONB is a poor prognostic indicator while the presence of mucous cells suggests a good prognosis.

In addition, we provided a review of the current literature regarding olfactory neuroblastoma and retropharyngeal lymph node metastasis from olfactory neuroblastoma.

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(PMID = 16691416.001).

[ISSN] 0937-4477

[Journal-full-title] European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] [Olfactory neuroblastoma in an old patient. Report of a case].

Olfactory neuroblastoma or esthesioneuroblastoma is a rare embrionary tumor, much more in old patients, which uses to present as a pollipoid mass with nasal obstruction, epistaxis and anosmia of long evolution.

We report the case of a 82-year-old male with such symptoms diagnosed by biopsy as neuroblastoma class III according the histologic Hyams grading.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

The authors describe a clinical case of extensive olfactory neuroblastoma, comprehensive the nasal cavity, paranasal sinuses and orbit who was treated in Otolaryngology Department of the Regional Hospital in Tarnów.

Diagnostic difficulties in olfactory neuroblastoma recognition and value of the immunohistochemical examinations were emphasized.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

Olfactory neuroblastoma is an uncommon neoplasm.

Typically, these tumors are indolent with long-standing symptomatology, but the fact that the lesions are indeed malignant has been proven by the repeated demonstration that they can metastasize to distant organs.

In this study, we collected 16 olfactory neuroblastomas from the Kaohsiung Medical University Hospital.

The aim was to investigate the value of immunoexpression of hTERT and cyclin-D1 in correlation with clinicopathologic features of olfactory neuroblastoma.

In conclusion, high hTERT expression can be considered a potential indicator of aggressive olfactory neuroblastoma.

[Publication-type] English Abstract; Journal Article

[Publication-country] China

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Cytopathologic features of olfactory neuroblastoma.

BACKGROUND: Olfactory neuroblastoma (ONB) is an uncommon neoplasm arising from the olfactory mucosa.

Because its cytopathology is largely limited to case reports, the goal was to evaluate a series of ONB cases, compare them with previously reported cases, and with a control group of pulmonary and cutaneous small cell neuroendocrine carcinoma (NEC).

METHODS: Six fine-needle aspiration (FNA) biopsies of metastatic ONB and one case with imprint smears of primary ONB were recovered from files.

Aspirates from seven FNA cases of metastatic pulmonary small cell NEC and four cases of metastatic Merkel cell carcinoma to the head and neck functioned as a control group and were compared with those of ONB.

We reported two Korean women who suffered from ectopic ACTH syndrome (EAS) caused by olfactory neuroblastomas.

The first patient was a 66-year-old woman who had been diagnosed as olfactory neuroblastoma and refused the management two years before and the second patient was a 37-year-old woman on chemotherapy for olfactory neuroblastoma.

OBJECT: Treatment of esthesioneuroblastoma (olfactory neuroblastoma) has been considerably improved by microsurgical techniques.

Taking into account the rarity of the disease the present study comprises a relatively large series of patients treated in a similar manner.

This series comprises 12 newly diagnosed esthesioneuroblastomas.

Paranasal and nasal endoscopic sinus surgery was performed.

All patients complained of nasal discharge and crusts.

CONCLUSIONS: Based on the favourable results recorded so far, the combination of endoscopic sinus surgery and radiosurgery can be considered a promising treatment option for esthesioneuroblastoma that merits further consideration.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Primary olfactory neuroblastoma originating from the inferior meatus of the nasal cavity.

Olfactory neuroblastoma is a rare malignant tumor of the nasal cavity arising from the olfactory neuroepithelium.

It usually presents as a polypoid mass high in the nasal vault including the cribriform plate, superior turbinate, and superior portion of nasal septum.

An 89-year-old man visited our office with symptoms of occasional left nasal bleeding and obstruction.

Biopsy was performed, and the diagnosis of olfactory neuroblastoma was confirmed by histopathologic examination and immunohistochemical staining.

The tumor was resected via medial maxillectomy, and the final pathologic report established that it was primary olfactory neuroblastoma that occurred from the inferior meatus.

In this report, a literature review was performed on the pathologic characteristics and prognosis of the tumor, and possible hypotheses that olfactory neuroblastoma has originated from the inferior meatus were discussed.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] [Retrospective analysis of the effect of intranasal endoscopic resection of olfactory neuroblastoma].

OBJECTIVE: To investigate the effectiveness of intranasal endoscopic resection of olfactory neuroblastoma and the significance of assistant radiotherapy.

METHODS: Six patients (4 men and 2 women) ranging in age from 9-68 years (median age, 48 years) with olfactory neuroblastoma treated endoscopically at the Third Affiliated Hospital of SUN Yat-sen University between August 2001 and September 2005 were retrospectively analyzed.

CONCLUSIONS: With simple approach, excellent visualization, a less operative invasiveness and no scar on face, the endoscopic approach appears to be the method of choice for the treatment of olfactory neuroblastoma.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title]Olfactory neuroblastoma causing ectopic ACTH syndrome.

Although no pituitary mass was detected by MRI of the brain, inferior petrosal sinus sampling showed a step-up of central to peripheral ACTH levels; these data are consistent with the diagnosis of Cushing's disease.

Ten months later, a mass was detected in the ethmoid sinus, which was surgically removed.

After resection of the ethmoid sinus tumor, her Cushingoid features and hypercortisolemia disappeared, but recurred after enlargement of a second mass in the maxillary sinus.

After resection of the maxillary sinus tumor, her hypercortisolemia subsided.

Histologically, the tumor tissues from both the ethmoid and maxillary sinus were identical and consistent with the diagnosis of olfactory neuroblastoma.

Thus, this is the second rare case with ectopic ACTH syndrome caused by olfactory neuroblastoma thus far reported.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

A pituitary MRI was performed, which revealed a normal pituitary; however, a large mass was seen centered in the ethmoid and paranasal sinuses with a significant amount of extension into surrounding structures.

A biopsy was performed and pathology of the specimen was consistent with esthesioneuroblastoma.

Immunohistochemical staining further defined the tumor as an ACTH-secreting esthesioneuroblastoma.

This case demonstrates the successful diagnosis and treatment of a rare neoplasm.

Ectopic ACTH syndrome due to esthesioneuroblastoma is extremely uncommon with only five other cases being discussed in the literature.

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[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

Three patients with olfactory neuroblastoma (ONB) of the nasal and/or paranasal cavity were treated with a combination of conventional radiotherapy (RT) and hypofractionated stereotactic radiation therapy (SRT).

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

The case of a patient with recurrent esthesioneuroblastoma complicated by ectopic adrenocorticotropic hormone production is presented, including the workup and management of this uncommon complication of an uncommon disease.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

OBJECTIVE: In a retrospective analysis we discuss our experience in the treatment of olfactory neuroblastoma (ON), a rare tumor of the sinonasal tract.

STUDY DESIGN AND SETTING: Nine patients with olfactory neuroblastoma were treated at the Bolognini Hospital of Seriate and at the Department of Otolaryngology of "La Sapienza" University of Rome combining endoscopic surgery and postoperative radiation therapy.

RESULTS: All patients, over an average follow-up which ranged from 26 through 60 months, are actually alive with non evidence of disease (NED).

CONCLUSIONS: Despite the short follow-up in this small series of patients, we suggest endoscopic surgery combined with postoperative radiation therapy as an alternative to the conventional modalities of treatment for esthesioneuroblastoma in selected cases.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

BACKGROUND: The efficacy and safety of chemotherapy with irinotecan plus docetaxel were retrospectively evaluated for olfactory neuroblastoma.

METHODS: Twelve patients with histologically proven advanced or metastatic olfactory neuroblastoma were treated with chemotherapy with irinotecan plus docetaxel at the study institution between 2001 and 2005.

Of these, 7 patients with locoregional disease and no prior radiotherapy received irinotecan plus docetaxel followed by definitive radiotherapy, 1 with photon radiotherapy and 6 with proton radiotherapy, whereas 3 patients with distant metastases and 2 with locoregional disease who had received prior radiotherapy received irinotecan plus docetaxel only.

Of the 7 patients with locoregional disease also receiving definitive radiotherapy, the 2-year survival rate was 100% and 6 patients were alive at the time of last follow-up.

CONCLUSIONS: Chemotherapy for olfactory neuroblastoma with irinotecan plus docetaxel is safe and manageable.

Induction chemotherapy followed by definitive radiotherapy may represent a promising option for patients with locally advanced olfactory neuroblastoma.

[Publication-type] Case Reports; English Abstract; Journal Article

[Publication-country] Poland

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title]Esthesioneuroblastoma.

Esthesioneuroblastoma is a rare tumor for which the published literature does not provide an evidence-based consensus treatment approach.

Multimodality therapy including a combination of surgery and radiation appears to provide the best disease-free and overall survival.

Chemotherapy should not be used as single-modality therapy for initial treatment but may provide additional benefit when used in combination with radiation and surgery, particularly in advanced-stage disease.

Combination chemotherapy should be considered as initial therapy for unresectable tumors and metastatic disease, and as salvage therapy in disease recurrence.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Do intracranial neoplasms differ in Ollier disease and maffucci syndrome? An in-depth analysis of the literature.

OBJECTIVE: Ollier disease (OD) and Maffucci syndrome are closely related, very rare syndromes, that are both associated with multiple sites of chondrodysplasia.

All 18 NSNs in OD were of glial cell origin, but only 5 of 9 NSNs in Maffucci syndrome were glial; other tumors included pituitary adenoma, olfactory neuroblastoma, malignant chordoma, and spindle cell hemangioendothelioma.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

INTRODUCTION: Olfactory neuroblastoma is a very aggressive tumour with high rates of locoregional recurrence and distant metastasis.

In this study, we present our experience and treatment outcomes for salvage therapy in patients with recurrent olfactory neuroblastoma.

MATERIAL AND METHODS: We retrospectively analysed 17 patients treated for advanced olfactory neuroblastoma during the last 15 years.

RESULTS: The disease-free 5-year survival rate was 48% in all patients and 42% in the 17 patients with recurrence or distant metastasis.

In 15% of patients with a clinical stage N0 at initial diagnosis, nodal recurrence developed and was successfully treated with salvage therapy.

CONCLUSION: Complete surgical resection, including craniofacial resection and postoperative radiotherapy without elective neck dissection, is the preferred approach in the treatment of advanced olfactory neuroblastoma.

[Publication-type] Comparative Study; Journal Article

[Publication-country] Netherlands

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] Noncontiguous bilateral esthesioneuroblastoma: a case report.

Esthesioneuroblastoma is a rare malignant neoplasm arising from the olfactory epithelium.

This tumor has a tendency to originate from one side of the nasal cavity or paranasal sinuses with frequent extension into the contralateral nasal cavity through direct invasion.

A review of the literature reveals numerous case reports describing esthesioneuroblastoma with unilateral or bilateral involvement; however, there have been no known reports of bilateral noncontiguous (multifocal) esthesioneuroblastoma.

We present a unique case of a patient diagnosed with two separate primary esthesioneuroblastomas who was managed with preoperative radiation followed by surgical resection of the tumor.

This case illustrates that esthesioneuroblastoma must remain as part of the differential diagnosis for a multifocal, noncontiguous intranasal and paranasal sinus mass.

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[Title]Esthesioneuroblastoma: a case report.

We report a case of esthesioneuroblastoma involving bilateral nasal cavity leading to bilateral nasal obstruction, epistaxis and proptosis of the right eye associated with decreased visual acquity on that eye and loss of smell.

A diffuse nontender, 6x6 cms swelling with illdefined margins was seen over the nasal bridge, extending superiorly to glabella and laterally to right maxillary region.

X-ray PNS showed soft tissue mass in the nasal cavity with destruction of nasal septum, intense periosteal reaction with destruction of right maxillary wall and extension to right orbit.

METHODS: A 39-year-old woman presented with a large tumor of the nasal cavity.

After the laboratory examinations, the tumor was classified as a Kadish stage A olfactory neuroblastoma.

CONCLUSIONS: Large olfactory neuroblastomas filling the entire nasal cavity extending back to the postnasal space can be completely removed using a transfacial approach as the Altemir technique modification is.

[Publication-country] Spain

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title]Olfactory neuroblastoma: a review and update.

Olfactory neuroblastoma (ONB) is a malignant neuroectodermal tumor that typically occurs in the superior nasal cavity.

In addition, problems in sampling, preservation, and clinical localization may make the diagnosis more challenging.

A large group of tumors may show morphologic overlap with ONB, with some mimicking low-grade tumors, whereas others mimic high-grade tumors.

This differential diagnosis has important treatment and prognostic implications.

Further complicating the issue is that many of the mimics themselves may show great morphologic and immunophenotypic variability.

This manuscript will review the typical clinical and pathologic features of ONB, address the great variability of ONB in our experience, will focus on the differential diagnosis and report on recent findings in these tumors including molecular genetics where applicable.

Undifferentiated malignant round cell tumors of the sinonasal tract and nasopharynx comprise of aggressive malignant neoplasms with overlapping morphologic features, which yet are very different and can be identified as carcinoma, lymphoma, sarcoma, melanoma or olfactory neuroblastoma.

The use of a panel of immunohistochemical markers and the identification, in some cases, of specific chromosomal translocations are most often required.

[Publication-country] Austria

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

[Title] [Esthesioneuroblastoma in children].

OBJECTIVE: To summarize the clinical characterization of esthesioneuroblastoma in children and adolescence.

METHOD: The esthesioneuroblastoma in children treated in our hospital from 1993 to 2004 were retrospectively evaluated, and thorough literature review of patients with esthesioneuroblastoma was performed by searching for the key word "esthesioneuroblastoma" in CHKD and in Pub Med between 1994-2004, also looking for its reference.

The main symptoms were nasal occlusion, proptosis, epistaxis, headache and the main location of disease were the intranasal, ethmoid sinus, orbital, anterior cranial fossa.

CONCLUSION: Esthesioneuroblastoma in children is very rare and combined surgery and radiotherapy should be selected.

The 5-year OS of patients with modified Kadish stage A or B disease and those with stage C or D was 78.4% and 49.7% (chi(2) = 2.10, P = 0.15), and the 5-year DFS was 47.1% and 38.4% (chi(2) = 0.08, P = 0.78), respectively.

CONCLUSION: Esthesioneuroblastoma is more prevalent in the young male adults than female, usually with locally advanced stage lesion when first diagnosed.

Radiotherapy plays an important role in the management of the disease.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

Esthesioneuroblastoma is a rare neuroectodermal tumor of the nasal vault with an aggressive biological behavior that is characterized by local recurrence, atypical distant metastasis, and poor long-term prognosis.

We report two cases of primary metastatic and locally recurrent disease, which were treated with computed tomography-guided interstitial high-dose-rate brachytherapy in palliative and curative intent, respectively.

Computed tomography-guided interstitial high-dose-rate brachytherapy should be considered as a feasible treatment option for advanced esthesioneuroblastoma.

[Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.

We report an EVN arising from the right frontal lobe near the olfactory tract in a 34-year-old male with worsening chronic epilepsy.

Our patient's tumour exhibited many uncommon features including ganglionic differentiation, increased mitotic activity and a high proliferative index.

We discuss the important differential diagnoses given the site of the tumour as well as the differentiating features from olfactory neuroblastoma, oligodendroglioma, anaplastic ganglioglioma and supra-tentorial primitive neuroectodermal tumour.

Interestingly, we found two novel, so far not described deletions, del(2)(q37) and del(21)(q22).

Applying SNP array karyotyping, we confirmed the chromosomal aberrations del(2)(q37.3), del(3)(q27.2), del(10)(q26.11), chromosomal imbalance on 17q, del(21)(q22), and revealed a number of so far unknown aberrations (gain of 2q14.3, 13q33.3, and 13q34).

While the cytogenetically revealed low frequency mosaic del(6)(q22q24) was not visible using SNP array karyotyping, some of the smaller imbalances (SNP array data) could not have been detected by classic cytogenetic analysis.